Regular ArticleHeterogeneous Intracellular Localization and Expression of Ataxin-3
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Development of an AAV-Based MicroRNA Gene Therapy to Treat Machado-Joseph Disease
2019, Molecular Therapy Methods and Clinical DevelopmentPhysiological and pathophysiological characteristics of ataxin-3 isoforms
2019, Journal of Biological ChemistryCitation Excerpt :These discrepancies may be explained by the isoform applied, the use of tags, and the localization within the protein as well as specific differences in the experiments like translational inhibition and inhibitor concentrations (80–88). Ataxin-3 was reported to be located in different compartments of the cell (3, 4, 46). Moreover, the nucleus was previously reported to be an essential site for MJD pathology (29, 30).
Experimental and Clinical Strategies for Treating Spinocerebellar Ataxia Type 3
2018, NeuroscienceCitation Excerpt :Atx-3, the affected protein, is a ubiquitously expressed and predominantly cytoplasmic protein. However, in certain cell types, atx-3 was also observed in both the nucleus and the cytoplasm (Paulson et al., 1997; Wang et al., 1997; Schmidt et al., 1998; Tait et al., 1998; Trottier et al., 1998; Pozzi et al., 2008; Macedo-Ribeiro et al., 2009; Reina et al., 2010). Atx-3 belongs to the Josephin family, which is one of five different families of deubiquitinating enzymes.
Toward understanding Machado-Joseph disease
2012, Progress in Neurobiology